Spontaneous hepatic repopulation in transgenic mice expressing mutant human α1-antitrypsin by wild-type donor hepatocytes

Journal of Clinical Investigation

Volumn 121, Issue 5, 2011, Pages 1930-1934

  Ding, Jianqiang ^a   Yannam, Govardhana R ^b   Roy Chowdhury, Namita ^a   Hidvegi, Tunda ^c   Basma, Hesham ^b   Rennard, Stephen I ^b   Wong, Ronald J ^d   Avsar, Yesim ^a   Guha, Chandan ^a   Perlmutter, David H ^c   Fox, Ira J ^e   Roy Chowdhury, Jayanta ^a  

^a ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

^b UNIVERSITY OF NEBRASKA MEDICAL CENTER   (United States)

^c UNIVERSITY OF PITTSBURGH SCHOOL OF MEDICINE   (United States)

^d STANFORD UNIVERSITY SCHOOL OF MEDICINE   (United States)

^e CHILDREN S HOSPITAL OF PITTSBURGH   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOVIRUS VECTOR; ALPHA 1 ANTITRYPSIN; MUTANT PROTEIN; SCATTER FACTOR;

ALPHA 1 ANTITRYPSIN DEFICIENCY; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; EMPHYSEMA; FEMALE; HEPATOCYTE TRANSPLANTATION; LIVER DISEASE; LIVER INJURY; MALE; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; TRANSGENIC MOUSE;

ALANINE TRANSAMINASE; ALBUMINS; ALPHA 1-ANTITRYPSIN; ANIMALS; BILIRUBIN; FIBROSIS; HEPATOCYTES; HUMANS; LIVER; MICE; MICE, INBRED C57BL; MICE, TRANSGENIC; MUTATION; TRANSGENES; TRANSPLANTATION, HOMOLOGOUS;

EID: 79955495937     PISSN: 00219738     EISSN: 15588238     Source Type: Journal    
DOI: 10.1172/JCI45260     Document Type: Article

References (16)

1. Crystal RG. α1-Antitrypsin deficiency, emphysema and liver disease: Genetic basis and strategies for therapy. J Clin Invest. 1990;85(5):1343-1352. (Pubitemid 20176425)
2. de Serres FJ. Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys. Chest. 2002;122(5):1818-1829.
3. Lomas DA, Evans DL, Finch JT, Carrell RW. The mechanism of Z α1-antitrypsin accumulation in the liver. Nature. 1992;357(6379):605-607.
4. Perlmutter DH. The role of autophagy in alpha-1-antitrypsin deficiency. A specific cellular response in genetic diseases associated with aggregationprone proteins. Autophagy. 2006;2(4):258-263. (Pubitemid 44342367)
5. The Alpha-1-Antitrypsin Deficiency Registry Study Group. Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin. Am J Respir Crit Care Med. 1998;158(1):49-59.
6. Dirksen A, et al. A randomized clinical trial of alpha(1)-antitrypsin augmentation therapy. Am J Respir Crit Care Med. 1999;160(5 pt 1):1468-1472.
7. Fox IJ, Roy-Chowdhury J. Hepatocyte transplantation. J Hepatol. 2004;40(6):878-886. (Pubitemid 38670119)
8. Overturf K, Al-Dhalimy M, Ou CN, Finegold M, Grompe M. Serial transplantation reveals the stem-cell-like regenerative potential of adult mouse hepatocytes. Am J Pathol. 1997;151(5):1273-1280. (Pubitemid 27479757)
9. Joseph B, Kapoor S, Schilsky ML, Gupta S. Bile salt-induced pro-oxidant liver damage promotes transplanted cell proliferation for correcting Wilson disease in the Long-Evans Cinnamon rat model. Hepatology. 2009;49(5):1616-1624.
10. Grompe M, Laconi E, Shafritz DA. Principles of therapeutic liver repopulation. Semin Liver Dis. 1999; 19(1):7-14. (Pubitemid 29220827)
11. Jiang J, et al. Correction of hyperoxaluria by liver repopulation with hepatocytes in a mouse model of primary hyperoxaluria type-1. Transplantation. 2008; 85(9):1253-1260. (Pubitemid 351670473)
12. Sifers RN, Carlson JA, Clift SM, DeMayo FJ, Bullock DW, Woo SLC. Tissue specific expression of the human alpha-1-antitrypsin gene in transgenic mice. Nucl Acids Res. 1987;15(4):1459-1475. (Pubitemid 17010428)
13. Hidvegi T, et al. An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis. Science. 2010; 329(5988):229-232.
14. Rudnick DA, et al. Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency. Hepatology. 2004;39(4):1048-1055. (Pubitemid 38428944)
15. Lindblad D, Blomenkamp K, Teckman J. Alpha-1- antitrypsin mutant Z protein content in individual hepatocytes correlates with cell death in a mouse model. Hepatology. 2007;46(4):1228-1235. (Pubitemid 350144787)
16. de la Cova C, Abril M, Bellosta P, Gallant P, Johnston LA. Drosophila myc regulates organ size by inducing cell competition. Cell. 2004;117(1):107-116. (Pubitemid 38469902)